{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T18:37:56Z","timestamp":1785350276721,"version":"3.55.0"},"reference-count":23,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,11,24]],"date-time":"2014-11-24T00:00:00Z","timestamp":1416787200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2015,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Thrombotic events in congenital hypo\u2010afibrinogenemia have been rarely reported, either in association or not with replacement therapy or thrombotic risk factors. We describe clinical findings and management of thrombosis of abdominal aorta with peripheral embolism in a patient with congenital afibrinogenemia. A review of arterial thrombosis in inherited hypo\u2010afibrinogenemia was also performed. The patient with a severe bleeding history requiring prophylaxis with fibrinogen concentrates (<jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content>) was admitted for ischaemia of the 4th right toe. An angio\u2010<jats:styled-content style=\"fixed-case\">CT<\/jats:styled-content> of abdominal aorta showed a thrombosis from the origin of renal arteries to the carrefour with a distal floating part. No thrombotic risk factors were found; a previous traumatic lesion of aortic wall might have triggered the thrombus formation, whereas the role of <jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content> prophylaxis remains uncertain. The patient was successfully treated with <jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content>, enoxaparin followed by fondaparinux, and low\u2010dose aspirin without bleeding or thrombosis recurrence. After 2\u00a0years, aortic thrombus was almost completely recovered. Sixteen hypo\/afibrinogenemia patients with arterial thrombosis were found in Literature, showing that thrombosis often occurs at a young age, involves large vessels, its recurrence is not unusual, and therapeutic strategy is not defined yet. Our therapeutic approach was effective and also safe, but further studies are needed to improve the knowledge of pathogenesis and the anti\u2010thrombotic management in this peculiar setting.<\/jats:p>","DOI":"10.1111\/hae.12507","type":"journal-article","created":{"date-parts":[[2014,11,25]],"date-time":"2014-11-25T03:07:56Z","timestamp":1416884876000},"page":"88-94","source":"Crossref","is-referenced-by-count":28,"title":["Thrombosis of abdominal aorta in congenital afibrinogenemia: case report and review of literature"],"prefix":"10.1111","volume":"21","author":[{"given":"S. M.","family":"Teresa","sequence":"first","affiliation":[{"name":"2nd Chair of Internal Medicine Department of Cardiac Thoracic and Vascular Sciences University Hospital of Padua Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Marta","sequence":"additional","affiliation":[{"name":"2nd Chair of Internal Medicine Department of Cardiac Thoracic and Vascular Sciences University Hospital of Padua Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. B.","family":"Emiliano","sequence":"additional","affiliation":[{"name":"2nd Chair of Internal Medicine Department of Cardiac Thoracic and Vascular Sciences University Hospital of Padua Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Mariangela","sequence":"additional","affiliation":[{"name":"2nd Chair of Internal Medicine Department of Cardiac Thoracic and Vascular Sciences University Hospital of Padua Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Raffaele","sequence":"additional","affiliation":[{"name":"2nd Chair of Internal Medicine Department of Cardiac Thoracic and Vascular Sciences University Hospital of Padua Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Z.","family":"Ezio","sequence":"additional","affiliation":[{"name":"2nd Chair of Internal Medicine Department of Cardiac Thoracic and Vascular Sciences University Hospital of Padua Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,11,24]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0033-1349222"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(13)70004-5"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1999.01681.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0030-1248720"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02841.x"},{"key":"e_1_2_6_7_1","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1055\/s-0037-1613195","article-title":"Inhibition of thrombin generation in plasma by fibrin formation (Antitrombin I)","volume":"88","author":"Bosh NB","year":"2002","journal-title":"Thromb Haemost"},{"key":"e_1_2_6_8_1","first-page":"850","article-title":"Fibrinogen\u2010independent platelet aggregation responsible of arterial thrombosis in an afibrinogemic patient","volume":"65","author":"Molho\u2010Sabatier P","year":"1991","journal-title":"Thromb Haemost"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.jvca.2004.11.021"},{"key":"e_1_2_6_10_1","first-page":"44","article-title":"Afibrinogenemia and gangrene, A case report","volume":"13","author":"Goudier E","year":"2007","journal-title":"JIM Sfax"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.01939.x"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001721-199509000-00008"},{"key":"e_1_2_6_13_1","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1055\/s-0037-1614201","article-title":"Lepirudin therapy for thrombotic complications in congenital afibrinogenaemia","volume":"91","author":"Schuepbach RA","year":"2004","journal-title":"Thromb Haemost"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/0218492312455180"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830410111"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1177\/000331979304400913"},{"key":"e_1_2_6_17_1","first-page":"597","article-title":"Congenital afibrinogenemia. 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